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Diagnosis: Cryoglobulemic (Type I) Glomerulonephritis
Cryoglobulinemic glomerulonephritis (GN) refers to glomerular pathology in the clinical context of circulating cryoglobulins. Of note, it is not a morphological pattern or a specific immunohistochemical or electron microscopic finding that defines cryoglobulins, but a positive laboratory test, that is, immunoglobulins that reversibly precipitate at temperatures <37°C. Laboratory testing is challenging, especially the temperature-sensitive pre-analytical phase is prone to mistakes, producing falsely negative results (Menter T, Hopfer H. Renal Disease in Cryoglobulinemia. Glomerular Dis. 2021;1(2):92-104. [PubMed link]).
Type I cryoglobulinemic GN is a lesion caused by cryoglobulins composed of monoclonal immunoglobulins IgG, IgA, or IgM. It is classically caused by underlying lymphoproliferative diseases, and nephropathy occurs in about 30% of patients with type I cryoglobulinemia. The most common histology seen on light microscopy is membranoproliferative GN, and immunofluorescence microscopy shows deposits of a single monoclonal immunoglobulin component. Electron microscopy shows subendothelial, occasional intramembranous, and mesangial deposits which may have a short microtubular or short fibrillary substructure, often >30 nm in diameter. (Guo, S., Fogo, A.B. (2019). Cryoglobulinemic Glomerulonephritis, Type I. In: Trachtman, H., Herlitz, L., Lerma, E., Hogan, J. (eds) Glomerulonephritis. Springer, Cham. https://doi.org/10.1007/978-3-319-49379-4_38).
Cryoglobulinemic GN is more frequently found in mixed cryoglobulinemias (type II and III) and less frequent in type I.
In our case, a particular ultrastructural organization of the deposits was not evident. The patient had multiple mediastinal and abdominal lymph nodes and a lymph node biopsy showed plasmablastic lymphoma. A monoclonal IgG-kappa component was detected in serum and cryoglobulins were positive.
See the chapter: Amyloidosis... and cryoglobulinemia of our Tutorial.
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Bibliography
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- Zhang LL, Cao XX, Shen KN, Han HX, Zhang CL, Qiu Y, Zhao H, Gao XM, Feng J, Zhang L, Zhou DB, Li J. Clinical characteristics and treatment outcome of type I cryoglobulinemia in Chinese patients: a single-center study of 45 patients. Ann Hematol. 2020;99(8):1735-1740. [PubMedlink]
- Menter T, Hopfer H. Renal Disease in Cryoglobulinemia. Glomerular Dis. 2021;1(2):92-104. [PubMed link]
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